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      • 활로氏四徵을 隨伴한 完全內臟轉錯症 : 2例 手術報告 A Report of Two Cases

        李寧均,金完植,洪彰義,金昇煥 中央醫學社 1962 中央醫學 Vol.3 No.5

        Case 1 : A twenty - two years old cyanotic korean male was operated on for tetralogy of Fallot with caridio- pulmonary bypass. Irreversible ventricular fibrillation occurred, Autopsy findings confirmed the diagnosis of tetralogy of Fallot with situs inversus totalis. Case 2 : A two tyears old Korean boy was admitted with chief complaints of cyanosis and dyspnea. By X-ray examinations, EKG, and cardiac catheterization, situs inversus totalis with tetralogy of Fallot was diagnosed. End- to - side anastomosis was done between superior veua cava and pulmoary artey(Glenn's poperation) in the left hemithorax. Patient's cyanosis and dyspnea were relieved post-operatively. Both cases showed no Kartahener's syndromes. Association of tetralogy of Fallot with situs inversus totalis has to be published yet according to keith (1958). It is of interest to experience two cases of this anomaly in this institution during a relatively short period of time.

      • 內臟轉錯症隨伴 左心症 : 合倂 大血管轉錯症에 對한 手術 1例 Levocardia with Situs Inversus Viscerum:A complicated combination of anomalies

        李寧均,朴鍾善,崔秉茂,金升煥,洪彰義,姜錫麟 中央醫學社 1963 中央醫學 Vol.5 No.5

        A six years old Korean girl was admitted in February 1963, with cyanotic congenital heart disease. By auscultation grade I -Ⅱ systolic murmur was audible along the left sternal border without thrill. Remarkable laboratory findings were high degree of and hematuria, polycythemia, albuminuria, and right sided hemiparesis. Transposition of the abdominal viscera with levocardia was found on routine X-ray study ECG showed right axis deviation and inverted P-wave in lead I . The right heart catheterization and angiocardiography with Shonander serial X-ray machine showed the right-sided (venous)atrium receiving the right and left superior vena cava separately, complete transposition of the great vessels, shunt at the ventricular level, and pulmonary stenosis. The inferior vena cava coursed along the left border of the spinal column and drained into the venous atrium, which is in the left hemithorax. The aortic arch was left-sided and bifurcated right ureter was visualized. Glenn' s procedure was done to increase the pulmonary blood flow in March 1963. Postoperative course was benign and uneventful. The follow-up study of the case, five month postively, revealed satisfactory results; increased exercise tolerance, marked relief from dyspnea on exertion, abolished cyanosis on rest, incresed arterial oxygen saturation (from 48% to 70%), normalization of hemogram, and increased pulmonary vascularity on the right lung field. The final anatomical findings of this unusually complicated cardiac anomaly were: (1) Levocardia with situs inversus viscerum, chamber inversion, and inverted P-wave in Lead I on ECG. (2) Transposition of the great vessels with pulmonary stenosis. (3) Intra-cardiac shunt or Shunts: i. Undetermined intracardiac shunt, Vntricular septal defect or single vent -ricle or atrioventricularis communis ? ii. Confirmed atrial septal defect. (4) Double superior vena cavae: left superior vena cava drained into the right atrial cavity (right atrium proper or coronary sinus?). Inferior vena cava on the left side. (By course of the cardiac catheter) Left aortic arch and left descending aorta. Bifurcated right ureter. Asplenia :presence not determined. Three lobes of the right lung confirmed by operation but numbr of lobes on the left lung undetermined. This report is believed to be the first operative palliation case of complete trans-position of the great vessels with pulmonary stenosis in a Korean literature.

      • 開放性動脈管 : 31例手術報告 Report of 31 Cases

        徐景弼,申漢壽,沈台燮,洪彰義,朱東雲,李寧均 최신의학사 1967 最新醫學 Vol.10 No.5

        Thirty one cases, 18 females and 13 males, were operated on for patent ductus arteriosus. There were three adult cases. Patent ductus arteriosus alone was present in 27, while in 4 there were significant associated cardiovascular anomalies. No deaths occurred in 24 patients isolated patent ductus a' eriosus without and with mild to moderate pulmonary hypertension. Severe pulmonary hypertension was present in 4 patients with an isolated patent ductus. An operative death occurred in the 2 patients in this group. One of them died as result of the tearing of the ductus during surgery. The remaining case died 3 days postoperatively., Patent ductus arteriosus with congenital cardiovascular anomalies was present in 4 patients. Single operative death occurred 7 days postoperatively because of bronchopneumonia of right lung and atelectasis of the left lung. There was an increased mortality when patent ductus arteriosus was associated with severe pulmonary hypertension. All survival patients showed very satisfactory postoperative results.

      • KCI등재

        Adult-Onset Genetic Leukoencephalopathies With Movement Disorders

        Fu Mu-Hui,Chang Yung-Yee 대한파킨슨병및이상운동질환학회 2023 Journal Of Movement Disorders Vol.16 No.2

        Genetic leukoencephalopathies (GLEs) are a group of white matter abnormalities with heterogeneous radiological and phenotypic features. Although these conditions have mostly been described in children, adult-onset cases are increasingly recognized owing to the widespread use of neuroimaging and advances in molecular genetic testing. The disease course is often progressive with a varied spectrum of presentations, trapping neurologists in the dilemma of differential diagnosis. Movement disorders are among the most common symptoms, and their diversity makes diagnosis challenging. In this review, we focus on adult-onset GLEs with movement disorders and offer a step-by-step diagnostic approach by clarifying the phenomenology of movement, advising investigations for acquired causes, describing the clinical and radiological clues to each disease, emphasizing the limitations of advanced molecular testing, and discussing the future application of artificial intelligence. We provide a list summarizing the leukoencephalopathies associated with different categories of movement disorders. In addition to guiding clinicians on how to narrow the list of differential diagnoses with the tools currently available, another aim of this review is to emphasize the inevitable trend toward applying advanced technology in diagnosing these difficult diseases.

      • 심근세포 및 내피세포의 분화에 미치는 Dimethyl Sulfoxide의 영향

        이동협,박이태,한승세,이융창 영남대학교 의과대학 1988 Yeungnam University Journal of Medicine Vol.5 No.2

        효소와 기계적인 힘을 이용하여 얻어진 심장세포를 심근세포와 내피세포를 분리하여 48시간 배양한후 양군으로 나눠서 관찰하였다. 실험군은 배양중에 10% DMSO에 1시간 처리하였고 DMSO를 처리하지 않고 계속 배양한 것을 대조군으로 하였다. DMSO효과를 관찰하기 위하여 도립현미경하에서 세포의 형태, 수축능력, 증식 능력등을 관찰하였고, 심근세포에서는 succinate dehydrogenase 반응으로 사립체의 증감을 대조군과 비교하였고, 심장내피세포에서는 thiamine pyrophosphatase의 반응으로 Golgi의 양을 대조군으로 비교하였다. 실험결과를 요약하면 다음과 같다. 1. DMSO에 의하여 심근세포는 분화된 형태로 모양이 길어졌고 사립체의 증가에 의해 succinate dehydrogenase의 반응이 대조군보다 강하게 나타났다. 2. DMSO에 의해 심근세포는 근세섬유의 파괴와 혼란이 초래되었고 이로 인하여 수축능력이 감소되었다. 3. 심장내피세포는 DMSO에 의해 세포증식이 감소되고 고유기능이 강조되어 Gologi의 표지효소인 thiamine pyrophosphatase의 반응이 대조군보다 강하였다. 4. 심장내피세포는 DMSO의 세포접착력 소실과 운동성 소실작용에 의해 배양중 많은 세포의 손실이 초래되었다. To elucidate the effects of dimenthyl sulfoxide on myocardial and endothelial cells in culture, the cells were exposed to 10% dimenthyl sulfoxide in culture medium for 1 hour at 48 hours after cell isolation. The general morphology and the cytochemical reaction of marker enzymes for mitochondria and Golgi complexes were investigated. The results were summarized as follows : 1. DMSO induced elongation and narrowing of the cells and increase of mitochondrial reaction in myocardial cells. 2. DMSO induced destruction and disruption of myofibrils in myocardial cells resulting in increase of contractile activities. 3. In the endothelial cells, DMSO suppressed proliferative activities but thiamine pyrophosphatase reactions were enhanced indicating increase of Golgi complex activity. 4. DMSO seemed to hamper with the adhesiveness and motility of the endothelial cells causing the decrease of the number of cells in vitro.

      • 동방결절에서 Ouabain에 의하여 발생하는 일과성 내향전류(TI)에 관한 연구

        최정연(Choi, Jung-Yun),홍창의(Hong, Chang-Yee),엄융의(Earm, Yung-E) 대한생리학회 1985 대한생리학회지 Vol.19 No.2

        Transient inward current (TI) was studied by the two micro-electrode voltage clamp technique in the sinoatrial node of the rabbit. The author confirmed that in 10<sup>-6</sup> M ouabain TI was found in the SA node and investigated the effects of ions, (Na<sup>+</sup>, K<sup>+</sup>, Ca<sup>2+</sup>, β-agonist (isoprenaline), local anesthetics (quinidine, lidocaine) and Ca-blockers (Co<sup>2+</sup>, verapamil, diltiazem) on the TI recorded during depolarizing voltage clamp pulses to -40 and -20 mV. The results obtained were as follows ; 1) 10<sup>-6</sup> M ouabain increased the frequency of sinus action potential and decreased the amplitude, especially overshoot of action potential. TI was induced by the depolarizing voltage clamp Pulses and the magnitude of the slow inward current (isi) decreased and the time course was slowed by the same depolarizing pulses. 2) 30% Na<sup>+</sup> and 24mM K<sup>+</sup> decreased by 10<sup>-6</sup> M ouabain and 6 mM Ca<sup>2+</sup> and 10<sup>-7</sup> M isoprenaline increased TI, i<sub>si</sub> and current oscillations. 3) Quinidine 5 X 10<sup>-7</sup>M) reduced TI and i<sub>si</sub> but lidocaine (10<sup>6</sup> -10<sup>-5</sup> M) didn t reduced or increase TI. Current oscillations increased and isi decreased by lidocaine. 4) Ca-blockers decreased the amplitude and the frequency of sinus action potential. TI and i<sub>si</sub> decreased significantly but were not abolished completely at the concentrations used in this experiment. Verapamil and diltiazem had inhibitory action on TI in 2 X 10<sup>-7</sup>M concentration and showed very slow recovery after wasting out with normal Tyrode solution.

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